Paraproteinemic hemoblastoses

Paraproteinemic hemoblastoses are a special group of lymphatic tumors in which tumor cells (lymphocytes or plasma cells) synthesize immunoglobulin. Since these leukemias, like others, arise from one initially altered cell, the whole mass of tumor cells produces some one immunoglobulin, which, even remaining normal, turns out to be useless or harmful for the body as a whole due to its extreme redundancy. As a rule, the synthesis of other immunoglobulins is reduced; Gradually increases the immunological failure of the body. Paraproteinemic hemoblastosis includes myeloma, Waldenstrom's macroglobulinemia, and heavy chain disease. These leukemias with their cellular substrate have mostly mature elements - plasmocytes, lymphocytes, and therefore should belong to the group of chronic leukemias. In addition, the ability to synthesize the same type of monoclonal (ie, produced by cells originating from one initially altered cell) immunoglobulin has the following tumors of the hematopoietic system: acute plasmablastic leukemia, rare forms of lymphosarcoma, and rare forms of cell-mediated lymphatic extramedullary neoplasms-lymphocyte.

Myeloma (multiple myeloma, plasmacytoma) is a bone marrow tumor consisting of plasma cells of equal maturity. The release of plasma cells into the peripheral blood is not characteristic for this leukemia, although it is sometimes possible.

The clinical picture at the onset of the disease does not have typical features. As the process progresses, pain occurs in the vertebrae, radicular syndrome, and pain in the ribs caused by the destruction (nested or diffuse) of the bones of the growing plasmacytoma. Sometimes the leading signs are associated with the oppression of bone marrow products: anemia, granulocytopenia with its infectious complications, thrombocytopenic hemorrhagic syndrome. In other cases, the leading symptom is rapidly growing proteinuria, renal failure.

The pathogenesis of the phenomena described is different. Bone destructive process is caused by osteolytic influence on the bone of tumor lymphocytic precursors of myeloma cells. The inadequacy of production of normal immunoglobulins is caused by the suppression of normal lymphocytopoiesis by tumor cells. At the heart of renal pathology is the deposition in the tubules and glomeruli of the excess monoclonal immunoglobulin or its derivatives. On this basis, sclerotic processes develop in the kidneys with progressive insufficiency. Protein deposits in the tissues of the internal organs, joints can provoke the development of the corresponding pathology, in particular heart failure, rheumatoid-like syndrome, etc. The suppression of the production of normal immunoglobulins leads to insufficient production of antibodies, a tendency to infections. Excess protein production can lead to the appearance of a syndrome of increased viscosity (severe asthenic syndrome, circulatory disorders of the microcirculatory type, thrombosis, infarctions of internal organs, hemorrhagic syndrome by the type of vasculitis).

The diagnosis is established based on the detection of a high content of plasma (sometimes atypical) cells in the bone marrow (above 15%), the appearance of monoclonal immunoglobulin in serum (often in the urine) in the form of a narrow band on the electrophoregram (M-gradient), lowering the level of normal immunoglobulins (Given immunoelectrophoresis). In doubtful cases, for example, with nonsecretory myeloma, the absence of a high percentage of plasmocytes in the bone marrow, the diagnosis is established in a specialized institution. The following signs are suspicious: unmotivated increase in ESR over a number of years (consequence of excess gamma globulins in the blood), persistent proteinuria (not albuminuria, but globulinuria, established by the method of urine electrophoresis), manifestation of osteodestructive foci, anemia, leukocyte and thrombocytopenia. By itself, the diagnosis of myeloma is not an indication for cytostatic therapy; It is assigned by a specialist.

Treatment usually begins in the hospital, prescribing cytotoxic drugs - cyclophosphamide or sarcolysine along with prednisolone and methandrostenolone (nerobol) or retabolil. Sarcolysin is prescribed at 10 mg / day every other day or every day, 200-300 mg per course. During the entire course, prednisolone 10-20 mg / day is given, retabolil 50 mg IV once a week (or nerobol 10-15 mg daily). After the completion of the course, they switch to maintenance therapy with sarcolysin 10 mg once every 5-10 days. With renal insufficiency, sarcolysin becomes extremely dangerous, as it is excreted by the kidneys and a violation of its excretion leads to a sharp increase in the concentration of cytostatic in the blood with the development of deep pancytopenia. Instead of sarcolysin, you can use cyclophosphamide 400 mg every other day inside, in / in or / m (for a course of 8-10 g). Throughout the course, prednisolone and nerobol (or retabolil) are given in the same dose as in the course with sarcolysin. After the end of the course, supportive therapy is carried out (400 mg cyclophosphamide per day once every 5-10 days). There are other treatment regimens for myeloma, requiring specialist involvement. With a decrease in the level of leukocytes to 1000 in 1 μl, cytostatic cancellation is necessary. Local lesions often require radiotherapy.

Waldenstrom's macroglobulinemia is a bone marrow tumor consisting of lymphocytes or lymphocytes and plasmocytes and characterized by high production of monoclonal macroglobulin - IgM. On the hematological and clinical signs, especially at the onset of the disease, the process differs little from chronic lymphocytic leukemia. However, increased production of coarse protein leads to the development of a syndrome of increased viscosity. Characterized by multiple small thromboses, thrombocytopenia, hemorrhagic syndrome.

The diagnosis is established on the basis of lymphocytosis in the blood, bone marrow, an isolated increase in the IgM fraction on the immunoelectrophoregram. Frequent, but not mandatory, a symptom - an increase in lymph nodes, spleen. Increased production of macroglobulin often leads to the development of the syndrome of increased viscosity: severe weakness, lethargy, congestion (up to coma), intermittent deafness and paresis are possible.

Treatment. Cytostatic therapy with chlorbutin, cyclophosphamide in the same doses as in chronic lymphocytic leukemia. In the syndrome of increased viscosity - plasmapheresis (removal of plasma with replacement with a solution of albumin and plasma substitutes or normal plasma).